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Updated: Dec 31, 2025
Isolation and Characterization of Tumor-initiating Cells from Sarcoma Patient-derived Xenografts
Published on: June 13, 2019
Establishment and characterization of NCC-CDS2-C1: a novel patient-derived cell line of CIC-DUX4 sarcoma
Yuki Yoshimatsu1, Rei Noguchi1, Ryuto Tsuchiya1,2
1Division of Rare Cancer Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
Abstract:
CIC-DUX4 sarcoma (CDS), an aggressive soft tissue sarcoma, is characterized by a CIC and DUX4 rearrangement. It has a dismal clinical course and high metastatic rate and shows chemotherapy resistance; therefore, a novel therapeutic strategy is required. Patient-derived cell lines are indispensable tools for basic and preclinical research. However, only a few patient-derived CDS cell lines have been currently reported. Therefore, in this study, we aimed to establish and characterize a novel cell line of CDS. We successfully established the NCC-CDS2-C1 cell line by using surgically resected tumor tissue from a patient with CDS. The NCC-CDS2-C1 cells harbored a CIC-DUX4 fusion gene without insertion and exhibited rapid growth, spheroid formation, and invasion. We screened the antiproliferative effects of small anticancer agent compounds, which included FDA-approved anticancer drugs, on NCC-CDS2-C1 cells in comparison with those on the two previously reported patient-derived CDS cell lines, NCC-CDS1-X1-C1 and NCC-CDS1-X3-C1. The response profile of NCC-CDS2-C1 was similar to but distinct from those of the other cell lines for the small anticancer agent compounds. Therefore, we conclude that the NCC-CDS2-C1 cell line will be a useful tool for basic and preclinical studies of CDS.
Insights
Researchers established a new patient-derived cell line, NCC-CDS2-C1, for CIC-DUX4 sarcoma (CDS). This cell line aids in studying the aggressive cancer and testing new therapies due to its distinct drug response profile.
Area of Science:
- Oncology
- Cancer Biology
- Genetics
Background:
- CIC-DUX4 sarcoma (CDS) is an aggressive soft tissue sarcoma with poor prognosis and chemotherapy resistance.
- Novel therapeutic strategies are urgently needed for CDS.
- Patient-derived cell lines are crucial for preclinical research but are scarce for CDS.
Purpose of the Study:
- To establish and characterize a new patient-derived cell line for CIC-DUX4 sarcoma.
- To evaluate the antiproliferative effects of small anticancer agents on the new cell line.
Main Methods:
- A novel CDS cell line, NCC-CDS2-C1, was established from patient tumor tissue.
- The CIC-DUX4 fusion gene was confirmed in the cell line.
- Antiproliferative screening of small anticancer agents was performed on NCC-CDS2-C1 and two existing CDS cell lines.
Main Results:
- The NCC-CDS2-C1 cell line exhibited rapid growth, spheroid formation, and invasion.
- NCC-CDS2-C1 cells harbored the CIC-DUX4 fusion gene.
- The drug response profile of NCC-CDS2-C1 was similar yet distinct from previously reported CDS cell lines.
Conclusions:
- The established NCC-CDS2-C1 cell line is a valuable tool for basic and preclinical research in CIC-DUX4 sarcoma.
- This new cell line offers a unique model for understanding CDS heterogeneity and drug response.
- Further studies using NCC-CDS2-C1 can accelerate the development of novel therapeutic strategies for CDS.
